Children with congenital heart disease (CHD) who were once considered “inoperable” are increasingly living into adulthood with improved quality of life. By some accounts, there are now more adults than children living with CHD (1). This turning point reflects decades of improvement across multiple specialties. Because of these advances in pediatric cardiac care, there is an increasing population of patients presenting for surgical, radiological, obstetric, and various other invasive procedures requiring anesthesia, bringing with them the entire spectrum of CHD along with its unique challenges and opportunities.

Although the subject of anesthesia care for patients with CHD has been reviewed (2–4), in practice, clinicians still face innumerable questions daily for which there are few data. How should these patients be prepared for surgery? What monitoring and testing should these patients undergo? What anesthetic techniques are safest? Where should these patients recover? At which centers should these cases be performed? What qualifications should the teams caring for these children possess? Pivotal in addressing these questions is the matter of risk. Where, specifically, does the perioperative risk lie?

In this issue of the Journal, Faraoni et al. (5) provide us with some answers. Using data from the American College of Surgeons (ACS) National Surgical Quality Improvement Program (NSQIP) database, the authors present a modified case-control study of children with (n = 4,494) and without (n = 4,494) CHD undergoing noncardiac surgery. Control subjects without CHD were matched 1:1 with CHD cases using a propensity score, and the case population was stratified according to heart disease severity (minor, major, and severe). The authors express their outcomes as odds ratios for 30-day mortality, overall mortality, and for several adverse post-operative events. Their results show that mortality and adverse events for children with minor CHD were no different from those for children without CHD, whereas children with major and severe CHD experienced an increased risk of mortality, cardiac arrest, and reintubation when compared with matched control subjects.

Although the findings of Faraoni et al. (5) are consistent with the existing published data (6–14), several features set the current study apart. First and foremost, they drew their data from a much larger population, the ACS NSQIP, a national database with multiple collaborating sites and rigorous built-in quality controls. Second, they used standardized categories for CHD as defined by ACS NSQIP, as well as propensity score matching. Such standardization methods have recently been hailed as essential in bringing about advances in care for this population (14). Finally, they identified a CHD population that is not at increased risk relative to the general population, (i.e., those with minor CHD). This identification of a low-risk group provides some very useful information for practitioners. Given that hospital costs for children with CHD represent 23% of the global cost, whereas accounting for only about 4% of hospital discharges (15), this finding is important for daily clinical decision making and for larger, system-level decisions that may shape health policy.

The current study is not without limitations. First, the ACS NSQIP database does not provide specific data to permit comparison among type of facility, caseload, provider, or management system. Second, the ACS NSQIP classification of “major” CHD is clinically quite broad and heterogeneous. These are important limitations in light of findings by Faraoni et al. (5) as well as a recent publication from the ACS (16). The ACS and its Task Force for Children’s Surgical Care have called for a system to designate pediatric surgical centers as “basic, advanced, and comprehensive,” according to resources (17). As a result, patients with major or severe CHD may be relegated in the future to receive care specifically at select “comprehensive” pediatric care centers. Such a recommendation may be difficult for families with limited resources, which is a topic worthy of discussion.

As the care for these complex patients continues to evolve, and as more patients with CHD present for elective surgery and anesthesia, we anticipate that our understanding of surgical risk will come into sharper focus. This elegant study by Faraoni et al. (5) should be understood as a clarion call underscoring the need for additional research to develop evidence-based multidisciplinary guidelines for the management of this growing, high-risk, and diverse population.

Footnotes

↵∗ Editorials published in the Journal of the American College of Cardiology reflect the views of the authors and do not necessarily represent the views of JACC or the American College of Cardiology.

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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